Von Willebrand disease (VWD) refers to a genetic condition that is brought about as a result of defective or missing Von Willebrand Factor (VWF), a blood clotting protein. VWF binds a key blood clotting protein called factor VIII and platelets in the walls of blood vessels. The binding helps in the formation of a platelet plug in the clotting process. This disorder is named after Erik Von Willebrand, a Finnish physician who first described the condition in the 1920s.
There are quite a number of hospitals in the United States where patients can seek Von Willebrand Disease treatment. However, patients need to travel in order to access these healthcare facilities. Due to the fact that insurance plans cover medical treatments but not travel expenses, patients need to plan wisely so that they can keep the travel costs at the minimum.
If you already know the health facility that you want to go for Von Willebrand Disease treatment, consultation or second opinion, you should go ahead and plan for your travel. Alternatively, you can check our list of medical facilities that offer treatment for this condition in the country. Additionally, if you are unable to meet your travel expenses, you should check our directory of charitable organizations that help in arranging free travel for medical treatments.
About Von Willebrand Disease
This is the most common of bleeding disorders and affects about one percent of the population in the United States. The condition is carried on the chromosome 12 and affects both genders equally.
Symptoms of Von Willebrand Disease
Individuals suffering from VWD experience easy bruising, frequent nose bleeding, and too much bleeding after invasive procedures, such as surgery and tooth extraction. Women experience menorrhagia (heavy menstruation lasting longer than normal) and hemorrhaging after child delivery.
There are three types of Von Willebrand Disease. These types are based on quantitative and qualitative defects in VWF. The fourth type of the disease called acquired VWD is not genetic. Acquired VWD in adults comes about after an autoimmune disease diagnosis such as lupus, from certain cancer types or from heart disease. Certain medications can also cause acquired VWD. The disease will manifest in the following manner;
- Clots greater than one inch (2.5 centimeters) in the menstrual blood flow
- Requirement to change tampons or pads more frequently than hourly.
- Requirement to use more than one sanitary protection to control menstrual blood flow
- Anemic symptoms such as fatigue, tiredness and short breaths.
Causes of Von Willebrand Disease
This disorder is caused by an inherited abnormal gene that is responsible for the control of Von Willebrand factor (blood protein that is crucial in blood clotting process). When the person has low levels of this factor or does not function normally, platelets will not stick together in the right manner. They will also fail to attach themselves as they should to the walls of the blood vessels in case of an injury. This causes interferences in the blood clotting process and in other instances, unrestrained bleeding.
The VWF also binds factor VIII (that stimulates blood clotting). Many patients suffering from VWD also have low levels of this factor VIII. This factor is also involved in hemophilia (another genetic blood clotting disorder that affects mostly males).
There are rare chances that VWD can develop later in life (acquired VWD) as a result of another underlying medical condition.
Where to Go for Von Willebrand Disease Treatment
There are many treatment facilities for Von Willebrand Disease in the United States. These hospitals have the latest equipments and medical technology, competent doctors, support staff and medications that help them provide comprehensive treatment option to patients. Among the best of these facilities include;
- Seattle Children’s Hospital
Experts at Seattle Children’s Hospital’s Cancer and Blood Disorders Center is dedicated in diagnosing and treating blood conditions in children and young adults, including von Willebrand disease. This is in line with the facility’s goals of achieving the best outcomes possible for children and to ease the effects of the disease. The center works with the child, family and doctors to provide the child with the suitable care and services.
Treatment of Von Willebrand Disease
In order to treat VWD, a proper diagnosis must be done first. The doctor will request a medical health history in order to determine whether other family members have ever been diagnosed with a blood clotting disorder or have had symptoms. The doctor may also recommend tests that are aimed at an evaluation of clotting duration and the ability of the patient to form a clot. The patient may also undergo an assay (clotting factor test) as well as other tests that measure platelet function. A test to measure the amount of VWF in the plasma (antigen test) may be performed too. Individuals suffering from this condition will normally have less than 50 percent normal VWF in their blood plasma.
When the doctor confirms the condition, a test that determines the particular type of the condition is done.
Treatment for VWD is dependent on the severity and the type of the disease. Some of the treatment options include the following;
- DDAVP (desmopressin acetate). This is an artificial version of vasopressin hormone. The hormone stimulates the release of VWF from the cells thereby increasing factor VIII. This comes in two forms; nasal spray and injectable. DDAVP is antiduretic and therefore fluid restriction is recommended to avoid reduced sodium in the blood stream and development of hyponatremia. This option is effective in patients suffering from type 1 and subtypes of type 2 VWD.
- Baxalta’s Vonvendi. This is the first recombinant VWF invention that, unlike other products, contains only VWF. This treatment option is recommended to be used by patients 18 years and above.
- Replacement therapies. This treatment option involves the patient being infused with doses of prepared and concentrated factors of blood clotting that contain VWF and FVIII (such as Humate-P). These treatments are useful in all types of the condition. If DDAVP is not effective or not suitable for your cases, the doctor will recommend this. Vonvendi (another recombinant VWF product) is another replacement therapy.
- These can be useful in women for controlling excessive menstrual bleeding. This is because the estrogen hormones found in the pills can enhance levels of VWF and FVIII activity.
Von Willebrand Disease is a lifelong disorder and has no curative options. However, treatments can assist to prevent bleeding complications. The treatment options are dependent on the condition type, severity, responses to previous therapies and the presence of other conditions and medications used.
Risks Associated with Von Willebrand Disease
There are several risks associated with Von Willebrand Disease. These risks include the following;
- aortic stenosis
- consanguineous relationships
- lymphoproliferative disorders
- myeloproliferative disorders
- positive family history
Who Can Go for Von Willebrand Disease Treatment
Individuals who are experiences signs and symptoms associated with Von Willebrand Disease should go for diagnosis, treatment, consultation or second opinion. Additionally, those with family history of genetic blood disorders should also go for screening.